Signal Transduction Antibodies 2
Anti-BOLA3 Antibody (CAB15985)
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- SKU:
- CAB15985
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Signal Transduction
Description
| Antibody Name: | Anti-BOLA3 Antibody |
| Antibody SKU: | CAB15985 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB IHC |
| Reactivity: | Human, Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-68 of human BOLA3 (NP_997717.2). |
| Application: | WB IHC |
| Recommended Dilution: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | mouse stomach, mouse heart, mouse kidney, rat liver |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-68 of human BOLA3 (NP_997717.2). |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | MAAW SPAA AAPL LRGI RGLP LHHR MFAT QTEG ELRV TQIL KEKF PRAT AIKV TDIS GGCG AMYE IKIE |
| Gene ID: | 388962 |
| Uniprot: | Q53S33 |
| Cellular Location: | Secreted |
| Calculated MW: | 11kDa/12kDa |
| Observed MW: | 12kDa |
| Synonyms: | BOLA3, MMDS2 |
| Background: | This gene encodes a protein that plays an essential role in the production of iron-sulfur (Fe-S) clusters for the normal maturation of lipoate-containing 2-oxoacid dehydrogenases, and for the assembly of the mitochondrial respiratory chain complexes. Mutation in this gene has been associated with multiple mitochondrial dysfunctions syndrome-2. Two alternatively spliced transcript variants encoding different isoforms with distinct subcellular localization have been reported for this gene (PMID:21944046). |
| UniProt Protein Function: | BOLA3: Defects in BOLA3 are the cause of multiple mitochondrial dysfunctions syndrome type 2 (MMDS2). A severe disorder of systemic energy metabolism, resulting in weakness, respiratory failure, lack of neurologic development, lactic acidosis, hyperglycinemia and early death. Some patients show failure to thrive, pulmonary hypertension, hypotonia and irritability. Biochemical features include severe combined deficiency of the 2-oxoacid dehydrogenases, defective lipoic acid synthesis and reduction in activity of mitochondrial respiratory chain complexes. Belongs to the bolA/yrbA family. |
| UniProt Protein Details: | Protein type:Unknown function Chromosomal Location of Human Ortholog: 2p13.1 Cellular Component: extracellular region Disease: Multiple Mitochondrial Dysfunctions Syndrome 2 |
| NCBI Summary: | This gene encodes a protein that plays an essential role in the production of iron-sulfur (Fe-S) clusters for the normal maturation of lipoate-containing 2-oxoacid dehydrogenases, and for the assembly of the mitochondrial respiratory chain complexes. Mutation in this gene has been associated with multiple mitochondrial dysfunctions syndrome-2. Two alternatively spliced transcript variants encoding different isoforms with distinct subcellular localization have been reported for this gene (PMID:21944046). [provided by RefSeq, Dec 2011] |
| UniProt Code: | Q53S33 |
| NCBI GenInfo Identifier: | 78486586 |
| NCBI Gene ID: | 388962 |
| NCBI Accession: | NP_997717.2 |
| UniProt Secondary Accession: | Q53S33,G3XAB0, |
| UniProt Related Accession: | Q53S33 |
| Molecular Weight: | 11,569 Da |
| NCBI Full Name: | bolA-like protein 3 isoform 1 |
| NCBI Synonym Full Names: | bolA family member 3 |
| NCBI Official Symbol: | BOLA3 |
| NCBI Official Synonym Symbols: | MMDS2 |
| NCBI Protein Information: | bolA-like protein 3; bolA homolog 3 |
| UniProt Protein Name: | BolA-like protein 3 |
| Protein Family: | BolA-like protein |
| UniProt Gene Name: | BOLA3 |
| UniProt Entry Name: | BOLA3_HUMAN |
 | Western blot analysis of extracts of various cell lines, using BOLA3 antibody (CAB15985) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Enhanced Kit (RM00021). Exposure time: 90s. |
 | Immunohistochemistry of paraffin-embedded Human oophoroma using BOLA3 Rabbit pAb (CAB15985) at dilution of 1:50 (40x lens). |
 | Immunohistochemistry of paraffin-embedded Rat ovary using BOLA3 Rabbit pAb (CAB15985) at dilution of 1:50 (40x lens). |
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